Management of tracheobronchial obstruction in infants using metallic stents: long-term outcome

© 2015, Springer-Verlag Berlin Heidelberg. Results: Twelve balloon-expandable metallic stents were placed in the trachea (n = 10) and/or bronchi (n = 2) of 5 patients with a median age of 13 months (range 5–30 months). Etiology of the airway obstruction included congenital tracheal stenosis (n = 4), giant cervical and superior mediastinal lymphatic malformation with tracheobronchomalacia (n = 1). Seven complications were reported (3 patients developed granulation tissue, 2 patients had re-stenosis, 1 stent migrated, 1 stent fractured). All patients survived and were in good condition with a median follow-up of 16 years (range 11–18 years). Three patients weaned off ventilator and oxygen. Conclusions: Endoscopic stenting with metallic stent has satisfactory long-term outcome in treating infants with tracheobronchial obstruction. Introduction: Tracheobronchial obstruction, although uncommon in the pediatric age group, remains a challenging problem. We review the long-term outcome of endoscopic metallic stenting in infants with tracheobronchial obstruction. Materials and methods: Medical records of all pediatric surgical patients who underwent tracheobronchial metallic stenting in our center were reviewed retrospectively from 1996 to 2014. Patients’ demographic data, including etiology, associated anomalies and nature of obstruction were reviewed. Outcome measures include complications such as re-stenosis, granulation tissue, stent migration, fractured stent, maximal tracheal diameter achieved, weaning of ventilator and growth at interval follow-up.postprin

Fine Mapping of the NRG1 Hirschsprung's Disease Locus

The primary pathology of Hirschsprung's disease (HSCR, colon aganglionosis) is the absence of ganglia in variable lengths of the hindgut, resulting in functional obstruction. HSCR is attributed to a failure of migration of the enteric ganglion precursors along the developing gut. RET is a key regulator of the development of the enteric nervous system (ENS) and the major HSCR-causing gene. Yet the reduced penetrance of RET DNA HSCR-associated variants together with the phenotypic variability suggest the involvement of additional genes in the disease. Through a genome-wide association study, we uncovered a ∼350 kb HSCR-associated region encompassing part of the neuregulin-1 gene (NRG1). To identify the causal NRG1 variants contributing to HSCR, we genotyped 243 SNPs variants on 343 ethnic Chinese HSCR patients and 359 controls. Genotype analysis coupled with imputation narrowed down the HSCR-associated region to 21 kb, with four of the most associated SNPs (rs10088313, rs10094655, rs4624987, and rs3884552) mapping to the NRG1 promoter. We investigated whether there was correlation between the genotype at the rs10088313 locus and the amount of NRG1 expressed in human gut tissues (40 patients and 21 controls) and found differences in expression as a function of genotype. We also found significant differences in NRG1 expression levels between diseased and control individuals bearing the same rs10088313 risk genotype. This indicates that the effects of NRG1 common variants are likely to depend on other alleles or epigenetic factors present in the patients and would account for the variability in the genetic predisposition to HSCR

RET Mutational Spectrum in Hirschsprung Disease: Evaluation of 601 Chinese Patients

Rare (RVs) and common variants of the RET gene contribute to Hirschsprung disease (HSCR; congenital aganglionosis). While RET common variants are strongly associated with the commonest manifestation of the disease (males; short-segment aganglionosis; sporadic), rare coding sequence (CDS) variants are more frequently found in the lesser common and more severe forms of the disease (females; long/total colonic aganglionosis; familial)

Fluorescence-guided pediatric surgery: The past, present, and future

Fluorescence-guided surgery (FGS) is becoming a common practice as an intraoperative aid in both adult and pediatric populations. It makes use of fluorescent contrast agents to identify targeted structures intraoperatively for precise resection with the help of a near-infrared (NIR) light camera. A wide spectrum of contrast agents and imaging platforms has been developed throughout the years to enhance their use for intraoperative tissue differentiation. FGS is applied in pediatric surgery for various indications, including thoracic, gastrointestinal, hepatobiliary, urogenital, oncology, and vascular surgery. Despite the popularity of fluorescence-guided pediatric surgery, the current literature is limited. The present review offers an up-to-date appraisal of the development and current status of FGS in the field of pediatric surgery

Predictors for failure after Kasai operation

© 2015 Elsevier Inc. Aim of the Study The outcome of Kasai operation is not uniformly successful. This objective of this study is to identify risk factors that predict early failure of this operation. Methods A retrospective study was carried out for all patients who received Kasai operation between 1980 and 2012. Patients referred from other centers for liver transplantation because of failed Kasai operation were also recruited. Main results A total of 185 patients were reviewed. A total of 119 (64.3%) patients failed Kasai operation, whereas 86 (46.5%) were considered as cases of early failure (< 3 years post-Kasai). With univariate analysis, the presence of associated anomaly (RR: 1.90, 95%CI 1:45-2:36), operation with laparoscopic surgery (RR: 3.14, 95% CI: 2.39-5.42), delayed clearance of jaundice (RR: 1.89, 95% CI: 1.56-2.67), and repeated cholangitis (RR: 2.56, 95% CI: 1.39-3.42) were associated with adverse outcome, whereas the use of adjuvant steroid at post-operative period appeared to be protective (RR: 0.64, 95% CI: 0.29-0.93). With multivariate analysis, repeated cholangitis was an independent risk factor for early failure after Kasai operation (RR: 3.16, 95% CI: 1.83-4.62). Furthermore, it was also considered as a risk factor for failure after 3 year post-Kasai (RR: 2.07, 95% CI: 1.43-3.42). Conclusion Patients with above risk factors are prone to fail Kasai operation in an early stage and require more frequent monitoring. Prevention and aggressive management of cholangitis should be considered as measures to avoid disease progression in the early stage. Lastly, performing open Kasai operation and the use of adjuvant steroid are potentially beneficial.Link_to_subscribed_fulltex

Iatrogenic diaphragmatic hernia in paediatric patients

© 2015, Springer-Verlag Berlin Heidelberg. Background and methods: Iatrogenic diaphragmatic hernia in childhood is rare, with only scanty case reports available in the literature. It is a potentially life-threatening condition resulting in intestinal obstruction and bowel strangulation. In this study, we retrospectively reviewed four patients with iatrogenic diaphragmatic hernia following right hepatic trisectionectomy with caudate lobectomy and liver transplantation in a tertiary referral centre. The patient characteristics, clinical presentation and risk factors were assessed. Conclusions: Iatrogenic diaphragmatic hernia should be considered in differential diagnosis in paediatric patients presenting with intestinal obstruction or respiratory distress after hepatic resection or liver transplantation for early diagnosis and management.Link_to_subscribed_fulltex

Cytomegalovirus-associated colitis mimicking necrotizing enterocolitis – A near miss diagnosis of neonatal colonic stricture

Although cytomegalovirus (CMV) is a common congenital infection in neonates, most patients are asymptomatic. Gastrointestinal manifestation is unusual. In this report, we described a newborn with perinatal CMV infection presented with symptoms mimicking necrotizing enterocolitis. We hope to alert clinicians about this possible diagnosis when managing newborn gastrointestinal diseases